title: Sturge-Weber syndrome: a favourable surgical outcome in a case with contralateral seizure onset and myoclonic-astatic seizures.
abstract: Sturge-Weber syndrome is a neurocutaneous disorder classically characterized by the presence of facial port-wine stain and ipsilateral leptomeningeal angiomatosis. It is often associated with refractory epilepsy which requires surgical treatment. We present a case of a patient who initially presented with partial seizures of temporo-occipital origin, ipsilateral to the pial angiomatosis. During the course of the disease, the patient developed medically refractory epilepsy with partial seizures originating predominantly from the contralateral temporo-occipital area as well as myoclonic and myoclonic-astatic seizures. Resection of the occipital and temporal lobe affected by the pial angioma resulted in favourable outcome. Bilateral dysfunction observed in Sturge-Weber syndrome may result in an increased capability of focal discharges to generate synchronous epileptiform activity leading to an increased incidence of generalised seizures, most probably via a mechanism of secondary bilateral synchrony. [Published with video sequences].
authors.affiliation: Neuronal Networks Group, Neuropharmacology and Neurobiology, School of Clinical and Experimental Medicine, University of Birmingham, Birmingham, United Kingdom.
authors.forename: Premysl
authors.initials: P
authors.lastname: Jiruska
authors.forename: Petr
authors.initials: P
authors.lastname: Marusic
authors.forename: John G R
authors.initials: JG
authors.lastname: Jefferys
authors.forename: Pavel
authors.initials: P
authors.lastname: Krsek
authors.forename: Roman
authors.initials: R
authors.lastname: Cmejla
authors.forename: Vera
authors.initials: V
authors.lastname: Sebronova
authors.forename: Vladimir
authors.initials: V
authors.lastname: Komarek
mesh_terms: D001921:Brain / Q000503:physiopathology / Q000601:surgery*; D002648:Child; D004569:Electroencephalography; D004831:Epilepsies, Myoclonic / Q000503:physiopathology / Q000601:surgery*; D006801:Humans; D008297:Male; D013341:Sturge-Weber Syndrome / Q000503:physiopathology / Q000601:surgery*; D016896:Treatment Outcome